Neonatal Hearing Screening- Universal vs High Risk Screening- Experience in HK Dr. Barbara CC Lam Consultant Honorary Clinical Associate Professor Department of Paediatrics and Adolescent Medicine Queen Mary Hospital 8 October 2005

Introduction Severe congenital hearing impairment (HI) is an important handicap affecting 1-3 per 1,000 live birth The prevalence of moderate to profound hearing loss was 2 - 4 % of NICU infants Infants with risk factors accounts for less than ~ 50% of cases ~ 165 infants per year are born with significant HI in HK per year

Hearing is an important sensory input Auditory stimulus perceives in the first few months of life forms the basis of speech, linguistics and cognitive development The language ability, the social, emotional, comprehensive and motor development of HI are adversely affected

Early Intervention Improves Outcome Yoshinaga-Itano showed age of the diagnosis of HI was the only significant variable to affect the language skill of HI child. Several prospective studies showed that the prognosis for intellectual, language and speech development in the HI child can be improved significantly when the diagnosis is made early and intervention begins before 6 month of age The first year of life, especially the first 6 months is most critical Robinshaw 1995 Apuzzo & Yoshinaga-Itano 1995 Moeller 1996 & Yoshinaga-Itano 1998

Past Situation in Hong Kong Since 1978, Maternal and Child Health Centre (MCHC) provide ‘universal’ hearing screening using Behavioural Distraction Test for infants around 6-9 months Most HA birthing hospitals perform hearing test (BAEP) for high risk infants The usual age at diagnosis of hearing impairment especially for those without risk factors is usually at 2 year and most infants receive treatment and education after 2 years

Recent Change in Hong Kong Most realized the importance of early diagnosis and early intervention Some HA birthing hospitals pilot UNHS Maternal and Child Health Centre (MCHC) replace the distraction test by Infant Hearing screening ( AOAE) for babies registered before 2 months through the early infant hearing & surveillance programme since Aug 2003 There is a lack of a coordinated territory wide policy on hearing screening and the lack of territory wide registry with tracking and monitoring system

What is the most appropriate and cost effective newborn hearing screening programme for Hong Kong ?

Newborn Hearing Screening Strategies High risk screening Universal screening

High Risk Indicators America Academy of Paediatrics Joint Committee on Infants Hearing 1990 Family history of HI Congenital infection Craniofacial anomalies Low Birth weight (1500 grams or less) Severe neonatal jaundice

High Risk Indicators Ototoxic medications in toxic range Bacterial meningitis Severe depression at birth Mechanical ventilation for or > 5 days Syndrome known to include sensorineural and/or conductive hearing loss

High Risk Screening ~ 5 - 10% of all babies born will exhibit one or more of these indicators 2.5 - 10% of these infants confirmed to have permanent congenital hearing impairment (PCHI) At least 50% of infants with PCHI do not have any of the risk factors

High Risk Multicentre Hearing Screening 1 year Project in HK 1999-2000 Multicentre project involving 5 HA hospitals Two stage distortion products OAE Conventional auditory brainstem response for failed screening and babies with neurological abnormalities Chan KY et al (Sponsored by The Save Children Fund(HK)

Protocol of High Risk Infant Screening Project OAE (14-28 days) Repeat OAE (<2 wks) MCHC Distraction test at 6-9 months ABR ENT & Audiologist ED for intervention Family history, asphyxia, meningitis, NNJ, congenital infection, PPHN etc N Abn

Summary of High Risk Infant Screening Result Live Birth 19,922 Risk Factor 546 ( 2.7%) OAE 533 ( 97%) 2nd OAE 70 ABR 46 ( 8.6%) ENT 30 *Hearing Loss 22 ( 4%) Default 13 (2.4%) CNS risk Additional ABR 210 ( 39%) Pass OAE but failed ABR - 13 ENT 13 * Hearing Loss 2 ( 0.3 %) failed Default 54 failed

Distribution of Risk Factors

Multicentre High Risk Infants Hearing Screening Project 1.2 per thousand (24 out of 19,922 LB) have HI - high risk approach detect less than 50% of cases 2.4% of at risk infants have moderate to profound HI HI infants were identified before age 4-6 months and all of them had received appropriate intervention before age of 9 months

Multicentre High Risk Infants Hearing Screening Project OAE may miss some case with CNS defects No significant HI was detected at age 9 months in those infants who passed newborn screening Default rate for OAE is 2.4%, for ABR for those with CNS risk factors is 26% - screening best to be performed before hospital discharge & coupled with a robust tracking system

Criteria for Public Health Universal Screening:- Easy-to-use screen tests - high sensitivity to minimize unnecessary referrals The condition being screened for is not otherwise detectable by clinical means 3. Interventions are available to correct the condition once detected 4. Early screening, detection, and intervention result in improved outcomes 5. The screening program is documented to be in an acceptable cost-effective range

Universal Newborn Hearing Screening Congenital Hearing Loss fulfills the established Criteria for Universal Screening A universal newborn hearing screening with a coverage rate of more than 95% is a more vigorous approach to achieve the aim of identifying most, if not all babies with congenital hearing loss at an early age to enable timely habilitation

Universal Newborn Hearing Screening Hospital based vs Community based? Which screening tools ? AABR ? OAE ? OAE and AABR

UNHS Projects in Hong Kong I. First Pilot hospital based UNHS - 2 stage OAE ( TYH)- 1999 II. Multicentre hospital based UNHS - Comparing different screening devices and protocols - 2001 Pilot study of Community based UNHS by 2 stage OAE in 4 MCHC -2001 Regional UNHS -2 stage AABR and cost effectiveness study – 2003-2005

Pilot UNHS- 2 stage OAE in TYH OAE Screen OAE Screen OAE Screen Day 1-4 Day 5-14 Day 21-30 Infants 806 913 872 Screened Failure 59% 21% 3.9% Rate PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

Pilot UNHS- 2 stage OAE in TYH Bilateral hearing loss is 0.28 High parental acceptance of UNHS – Coverage rate is 99.3% Unacceptably high false positive rate ( 20%) in first 2 week due to ear debris Refer rate for diagnostic audiological test is 3.5% PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004 Questionnaires study on Parental Acceptance of Newborn Hearing Screening 91% consider newborn hearing screening desirable 82% favor pre-discharge screening 56% do not have a sound knowledge on hearing developmental milestone, undesirable to rely on parental self surveillance to detect HI in their babies PK Ng, Y Hui, BCC Lam et al HK Medical Journal 2004

Hospital based Multi-centre UNHS – Screening Device and Protocol 3 different screening protocols 2-stage AABR (Algo model 2e Color Newborn Screener) 2-stage AABR (Biologic 2 in 1 screener) 2 stage OAE-AABR (Biologic 2 in 1 screener) Pre-discharge screening

Hospital based UNHS- Comparisons for 3 Different Screening Protocols BWY Young, BCC Lam CM Wong et al 2002 AABR- OAE- ABear- AABR(Algo) ABear ABear( Bio) Failed first Test 5.3% 29.1% 15.1% Final Refer Rate 0.6% 5.0% 3.8% Average Screening 11 11 17.5 Time (min.) Disturbance score 1.21.7 0 0.7 1.5 1.8

Hospital-based Multicentre UNHS BWY Young, BCC Lam CM Wong et al 2002 High coverage rate of 95% - UNHS is feasible before discharge from birthing hospital The 2 stage AABR screening yielded the lowest refer rate - 0.6% The time spent in the 3 screening methods are comparable OAE screening cause the least disturbance to the babies, followed by AABR

Pilot Study of Community Based UNHS in MCHC KY Chan, SSL leung HKJ Paedatr 2004 4 MCHC in August 2000 to July 2001 Infants with first MCH attendance at 2 weeks to 2 months 2 Stage OAE, re-screened 2 weeks later if failed the first screening

Pilot Study of Community based UNHS in MCH Uptake rate 77% of attendees at MCH Overall coverage rate not known Failure rate: 2nd screen 3.8% Out of 3,949 screened, 4 cases of Bilateral HI - 1 per 1000 babies Mean age of HI identification 85 days (vs 9-12 month for distraction test) KY Chan, SSL leung HKJ Paedatr 2004

QMH & PYNEH Universal Newborn Hearing Screening A regional 2-stage AABR and cost effective analysis

UNHS Screening Protocol - 2-stage AABR – AABR All babies born in QMH & PYNEH Stable and >35 weeks No Yes Distribute information sheet when baby is stable and >35 weeks on the first day of life 1st AABR same / next day after newborn examination 1st AABR screening 2nd AABR Screening on the same / next day Discharge and encourage to attend MCHC for on going surveillance Pass Fail

UNHS Screening protocol - 2-stage AABR – AABR Counseling by Paediatrician Refer to audiologist for diagnostic BAER Paediatric follow-up appointment 4 weeks later Diagnostic BAER Follow-up once by paediatrician Follow-up by paediatrician Follow-up by ENT Surgeon Refer to Special Education Unit for early habilitation Pass Fail

Can be used in Nursery Environment ( 5-20 min)

UNHS- QMH & PYNEH 2 stage AABR- 2003-2005 Total birth 14,604 Babies screened 14,560 Coverage rate 99.7% Failed 2nd screen 1.5% Confirmed HI 76 (0.5%) Bilateral HI 36 Unilateral HI 40

UNHS- QMH & PYNEH 2 stage AABR- 2003-2005 Time Median 1st screening 22 hrs 2nd screening 41 hrs Diagnostic BAER 43 days ENT 66 days Special ed. 158 days Hearing aid 198 days

Recommended Parameters for Effective UNHS Parameters Study AAP High Coverage Rate 99.7%  95% Good Sensitivity (RR) 1.5%  4% Zero false negative   Bilateral HL  35dB   Before discharge  

Cost No. Per baby Cost Average cost/birth $ Equipment 2 109,161 18 Staff hours 5,098 20 min. 130-105/hr 40 Consumable 3,028 0.25 75 12 Total 70

UNHS: Cost Analysis Capital cost of equipment Manpower & consumable cost Cost for confirmatory test Life long quality of life Differences in life-time learning capabilities Education cost

Long term Cost analysis

Cost Effectiveness Long term cost analysis showed that the cost of UNHS could be offset by savings from reduced burden on special education Positive gain at 10-11 years after implementation.

Hospital vs Community Based - Coverage Rate Hospital based: TYH 2 stage OAE 98.9% LB QMH PYNEH 2 stage AABR 99.7% MCH based: Distraction test 60% at 9M 2 Stage OAE 72% of all attendees

Hospital vs Community Based High Sensitivity Recommended parameter - Refer rate for diagnostic test Target < 4% Refer rate for diagnostic test Hospital based AABR program 1.5% MCH based OAE program 3.8%

Hospital vs Community Based - Early Referral Recommended Parameter - Target 100% before 3 months or shortly after birth Mean age of referral for diagnostic test Hospital based AABR program - 41hours MCHC based OAE program - 54 days

Hospital vs Community Based - Parental Acceptance Screening test is completed before discharge Child back to China High parental satisfaction Less anxiety due to lower and earlier referral for diagnostic test

Hospital VS Community Based Cost Analysis Hospital based AABR MCH based OAE 8 centres ($M) 44 centres ($M) Capital cost 1.2 3.2 of equipment Annual labour cost 2.38 2.83 Annual consumables 2.5 0.8 Cost of diagnostic test 0.5 12 Cost per deaf child 0.33 0.95 identified

Available Supporting Facilities! Hospital based UNHS Available specialist support and expertise including audiologist, paediatrician and ENT surgeon for counseling and further audiological evaluation

Universal Hospital Based Predischarge Newborn Hearing should be Introduced Efficacy of a screening program Maximal coverage Good sensitivity (low refer rate) High specificity (low false negative rate) High patient acceptance Cost effective

The Way Forward - Collaborative model Combine the specific competencies of the 2 involved parties

Role of Community Centre Establish and maintain a central registry and monitoring system Establish and maintain a tracking program that monitor all referrals and miss To provide mop up service for out-of- hospital births Ongoing surveillance for late onset hearing impairments

Summary Our UNHS studies demonstrated comparable incidence of HI among local infants at 3 to 5 per thousand A hospital based UNHS ensure high coverage and early detection 2 stage AABR achieve a low refer rate , high parental acceptance at an acceptable costs

Summary Newborn hearing screening program Good screening method Tracking and follow up system Early identification and intervention Ongoing evaluation for late onset HI A territory wide hospital based UNHS should be implemented to enable early detection and intervention leading to improved language and learning outcome in HI children

Acknowledgement HA High Risk Infants Hearing Screening Program CB Chow KY Chan B CC Lam W Wong TF Fok CW Law WH Lee KC Wong KN Yuen CC Shek E Wong Multicentre Universal Newborn Hearing Project B Young BCC Lam Y Hui E Wong W Yeung T Wong M Tong CB Chow W Wong P Chan

Acknowledgement TYH pilot universal newborn hearing screening P K Ng Barbara Lam C Y Yeung Winnie Goh Yau Hui QMH & PYNEH Regional UNHS BWY Young BCC Lam W Yeung Y Hui D Au

Thank You !

Risk Indicates for Infants with Late Onset/Progressive Hearing Loss Neonatal risk factors including hyperbilirubinemia requiring exchange transfusion Syndromes associated with hearing loss such as Usher’s syndrome Neurodegenerative disorders such as Friedreich’s ataxia Head trauma Recurrent persistent otitis media with effusion for more than three months

Risk Indicates for Infants with Late Onset/Progressive Hearing Loss Caregiver concern about hearing, speech or language and/or developmental delay Family history of permanent hearing loss Stigmata associated with permanent hearing loss Postnatal infections associated with hearing loss, such as bacterial meningitis In-utero infections including cytomegalovirus, herpes, rubella, syphilis, and toxoplasmosis

Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement Screening Tracking and Follow Up Identification and Intervention Evaluation

Current Recommendation on Hearing Loss Universal detection of hearing loss in infants before 3 months Appropriate intervention no later than 6 month Targeted at permanent bilateral or unilateral, sensory or conductive hearing loss, average 30-40 dB or more in the frequency region important for speech recognition

Current Recommendation on Hearing Loss 4. Infants who pass NHS, but have risk indicators for auditory disorders should receive ongoing audiologic and medical surveillance and monitoring for 3 years 5. Infants and family rights are guaranteed through informed choice, decision marking and consent

JCIH Recommends Benchmarks for Screening, Identification and Intervention Within 6 months of initiation of a screening program must screen a minimum of 95% of infants during birth admission or before 1 month of age Referral rate for audiologic and medical evaluation after screening should be 4% or less within 1 year of the programme Document efforts to follow up on 95% of infants who do not pass the screen and actual FU of 70% or more of infants

More Cost Effective ! Cost Analysis- Assumptions Annual livebirths in HK 55,000 Annual livebirth in HA hospitals = 40,000 Private hospitals provide their own UNHS service Incidence of PCHI = 3 per 1,000 livebirths Annual PCHI cases = 165 No. of MCHC providing hearing screening service = 42 out of the 50 MCHCs No. of birthing hospitals in HK = 9 Gazette cost of ABR = $1,600

Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement 1) Screening Testing both ears, with a minimum of 95% coverage rate Able to detect significant bilateral HL of  35 dB in the better ear Low false-positive rate of  3% and low referral rate for further assessment of < 4% Ideally should have false-negative rate of zero

Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement 1) Screening Screening methods should be noninvasive, quick and easy to perform Screening should be conducted before discharge from the hospital whenever is possible Each birthing hospital should have an established UNHS program

2) Tracking and Follow Up Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement 2) Tracking and Follow Up Aim at 100% follow-up with a minimum of 95% successful follow-up Establish and maintain a central monitoring system for all hearing screening programs Establish and maintain a tracking program that monitors all referrals and misses

3) Identification and Intervention Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement 3) Identification and Intervention Aim at 100% of infants with significant congenital hearing loss being identified by 3 months of age and having appropriate and necessary intervention initiated by 6 months of age The Paediatrician should coordinate and ensure access for all affected children to appropriate expert services

4) Evaluation – ongoing and regular by central monitoring and tracking Recommended Parameters of an Effective UNHS Program - JCIH 2000 Position Statement 4) Evaluation – ongoing and regular by central monitoring and tracking The performance of UNHS programs Tracking and follow-up system Intervention services to ensure that sufficient expert services are available for affected children and the outcomes from interventions provided are effective

Distribution of Risk Factors for Moderate – Severe Hl Risk factors Moderate Severe Profound Auditory Hearing loss Hearing loss Hearing loss neuropathy Family history of deafness 1 (HA) Ear malformation 1 1 Cleft palate 1 Multiple craniofacial abn 1 Low birth weight 3 (2HA) 1 Severe asphyxia 1 (HA) Down syndrome 1 1 CHARGE association 1 (HA) syndrome Total 13, HA - Hearing aid (5)

Comparison of Language Development for the Screen and Non-screen HL Children Semin Neonatol Yoshinaga-Itano 2001 Screen Non-screen Identification by 6 month 84% 8% Language development 56% 24% Normal - LQ  80 No. of types of consonant 4.5 0 (50th %) Total no. of words (50th%) 95.5 14.5 No. of different words 30 7

Newborn Hearing Screening Milestones 1989 Project to assess the feasibility of screening infants in nurseries for HL (TEOAE & ABR) in the U.S.A. 1992 Consensus conference on NHS recommended that every newborn be screened in the 1st month of life 1993 Rhode Island was the first state to achieve universal newborn hearing screening

Newborn Hearing Screening Milestones APA published its policy statement ” newborn and infant hearing loss- detection and intervention” 1999 First pilot study of UNHS in TYH maternity hospital in HK 2000 JCIH position statements expand the concept to early detection and Interventions through integrated interdisciplinary systems of UNHS, evaluation and family centered interventions

Newborn Hearing Screening milestone 2003 U.S.A. 37 states have legislation mandating UNHS and all states are developing hearing screening systems 2002 Published studies of UNHS in Taiwan, Japan and China

Cost-effectiveness and Test-performance Factors in Relation to UNHS M Cost-effectiveness and Test-performance Factors in Relation to UNHS M. Gorga Research Review 2003 The costs of UNHS exceeds the benefits at the initiation of programme After 4 years of operation, UNHS program will result in a net savings to society The saving increase rapidly, reaching a maximum annual benefit of 7 billion dollars 75 years after initiation